EDITORIALS
REVIEW
Autism spectrum disorders (ASDs) are common neurodevelopmental disorders characterized by heterogeneity and high variability in severity of mental, neurological, and somatic symptoms. Such gastrointestinal malformations as abdominal pain, constipation, diarrhea, gastroesophageal reflux, nutritional disorders, intestinal microbiota disorders are common in autistic children. The association between the severity of gastrointestinal malformations and the severity of behavioral symptoms in ASDs was revealed. Early diagnosis and assessment of gastrointestinal malformations in ASDs is crucial for their further correction to optimize nutrition, to develop individual complex treatment and management with the involvement of multidisciplinary specialists.
This article describes various COVID-19 dermatological manifestations that can develop in children. Their incidence and clinical features are described. Chilblain-like lesions were considered as the most typical in children population after coronavirus infection. Description of skin manifestations in multisystem inflammatory syndrome in children is also presented, issues of laboratory diagnosis are covered as well.
CLINICAL RECOMMENDATIONS
Experts of the Union of Pediatricians of Russia have developed modern guidelines on management of children with atypical hemolytic uremic syndrome (aHUS). aHUS is ultra-rare (orphan) disease with progressive and relapsing course during the life with potentially life-threatening state. This article covers issues of disease etiology, pathogenesis, and classification. Specific attention is paid to the diagnosis, differential diagnosis, and therapy of aHUS according to the evidence-based approaches.
The problem of timely diagnosis and proper management of patients with cystic fibrosis is crucial not only in our country, but throughout the world. Experts of the Union of Pediatricians of Russia have considered various issues of etiology, pathogenesis, epidemiology, diagnosis, and treatment of this genetic disease in a modern light. Particular attention was paid to screening methods for early diagnosis of cystic fibrosis. The principles of complex therapy were justified, including rational use of antibacterial and mucolytic drugs and enzyme replacement therapy that significantly determine the disease prognosis.
For Pediatricians' Practice
Background. Students, as the most active and mobile part of population, often unite into educational and informal groups, move to other regions or countries, and present a specific risk group for the spread of new coronavirus infection. Thus, they require preventive vaccination. objective. The aim of the study is to study the immunological potency, tolerance, and efficacy of Gam-COVID-Vac vaccine among students of Krasnodar. Methods. 119 seronegative students (18-30 years old) were examined. SARS-CoV-2 IgG (ELISA method) was determined 1, 3, and 6 months after two completed rounds of vaccination. Post-vaccination adverse events and COVID-19 cases were evaluated in the study. Results. SARS-CoV-2 IgG level 1 month after vaccination ranged from 6.15 to 19.38 and was to 16.39 (AU/mL) ± 1,12. Immunological potency values ranged from 4.407 to 21.5 (AU/mL) (14.74 ± 2.93) 3 months after. IgG titers were in the range of 4.14 to 17.71 (AU/mL) (10.97 ± 4.69) 6 months after. Adverse events after vaccination were revealed in 34 respondents (28.6%). Among them, local (hyperemia, pain, edema) — 21 (17.6%): slight — 90.4%, major — 9.6%; general (fever, weakness, algor, headache, arthralgia, myalgia) — 13 (10.9%): slight — 69.2%, major — 30.8%. The increase in vaccination coverage in students from 30.3 to 79.1% reduced the COVID-19 morbidity from 3.81 to 1.57%. Conclusion. Gam-COVID-Vac vaccine induced stable humoral response, demonstrated sufficient safety, and reduced morbidity 2.4-fold.
SHORT REPORT
FROM THE UNION OF PEDIATRICIANS OF RUSSIA
NEWS
IN MEMORY OF
CASE REPORT
The article presents clinical case of Job syndrome, variant of primary immune deficiency disease confirmed genetically. This case is interesting for its early diagnosis due to comprehensive assessment of anamnestic, clinical, and laboratory data. Such typical phenotypic features as facial dysmorphisms, recurrent localized purulent infections, and laboratory parameters (absolute neutropenia and eosinophilia) were the major signs of autosomal dominant hyper IgE syndrome in a child with normal IgE levels. The combination of 2 heterozygous mutations in STAT3 gene inherited from his father and his mother has played its role in disease clinical features in the child.
Laryngeal traumas in children are quite rare due to the anatomical features and properties of cartilaginous tissue. However, we cannot rule out possible damage to the larynx in case of impact injury in the neck area. The symptoms can develop later, several hours after the injury up to a life-threatening state.
Background. Agammaglobulinemia is a disease from the group of primary immune deficiencies with impaired antibodies production characterized by significant decrease or complete absence of B-cells. Due to the absence of pathogenetic treatment, severe bacterial lesions of various organs and tissues can develop, and they can lead to the patient death. Timely history taking and clinical and laboratory data analysis will allow to reveal this pathology early and start the therapy that can save patient's life. Clinical case description. This article demonstrates clinical case of agammaglobulinemia late diagnosis with development of severe complications and reversal of clinical manifestations in response to pathogenetic therapy. Conclusion. Despite the disease onset in the second half-year period of life, the presence of recurrent purulent processes resistant to standard treatment and the significant decrease of all immunoglobulin levels, agammaglobulinemia was suspected only at preschool age. The outcomes were disabled patient, and considerable aggravation of the quality of life.
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